tbuckley/cabot-search huggingface dataset:
AB1510 THE RELATIONSHIP BETWEEN EMERGING NEW INFLAMMATORY MARKERS AND FAMILIAL MEDITERRANEAN FEVER SEVERITY
Authors: S. Acer Kasman, M. E. Tezcan, N. Şen, S. Ösken, S. Yilmaz-Oner
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Neutrophil lymphocyte ratio (NLR), platelet lymphocyte ratio (PLR) and monocyte lymphocyte ratio (MLR) are emerging inflammatory parameters. These parameters have been associated with disease activity and response to treatment in many rheumatological diseases. <h3>Objectives</h3> To evaluate the relation between these parameters and familial Mediterranean fever (FMF) disease severity. <h3>Methods</h3> We included 274 FMF patients in the study. First, we measured FMF disease severity cross-sectionally in patients with International FMF severity score (ISSF). We then divided the patients into mild disease (ISSF score ≤ 2) and moderate-severe disease (ISSF score &gt;3) according to ISSF scores. Finally, we compared the NLR, MLR and PLR values between the two groups. <h3>Results</h3> Of 274 patients, 70 (25.5%) had moderate-to-severe FMF disease. More patients had amyloidosis, and the frequency of male gender was higher in the moderate-to-severe group. Other demographic and disease-related characteristics were similar between the groups. NLR, MLR, and PLR values were also similar between groups (Table 1) <h3>Conclusion</h3> Although emerging inflammatory markers are popular in the field of rheumatology, these parameters were unable to differentiate disease severity in patients with FMF. <h3>References</h3> [1]Erer, B., E. Demirkaya, S. Ozen, and T. Kallinich. What is the best acute phase reactant for familial Mediterranean fever follow-up and its role in the prediction of complications? A systematic review. Rheumatol Int 2016; 36(4): 483-7. [2]Gasparyan, A.Y., L. Ayvazyan, U. Mukanova, M. Yessirkepov, and G.D. Kitas. The Platelet-to-Lymphocyte Ratio as an Inflammatory Marker in Rheumatic Diseases. Ann Lab Med 2019; 39(4): 345-357. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.
AB0332 APPLICATION IN CLINICAL PRACTICE OF THE SCREENING CRITERIA FOR INTERSTITIAL LUNG DISEASE IN RHEUMATOID ARTHRITIS PROPOSED BY SER-SEPAR IN A COHORT OF RHEUMATOID ARTHRITIS. PRELIMINARY STUDY
Authors: H. Borrell Paños, E. Trallero Araguas, X. Michelena, A. Pluma Sanjurjo, L. Coronel Tarancon, D. V. Clofent Alarcon, H. F. Avalos Bogado, S. Marsal, M. Lopez Lasanta
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> A screening tool has recently been proposed by two scientific societies (Spanish Society of Rheumatology and Pneumology), combining clinical and serological data to improve the detection of interstitial lung disease (ILD) in rheumatoid arthritis(RA) [1]. <h3>Objectives</h3> To apply a new ILD screening tool in a cohort of patients with RA in order to determine its prevalence, and evaluate the burden of complementary exams generated for this purpose derived from its thorough application. <h3>Methods</h3> Observational and single-center cross-sectional study of a cohort of RA patients under active follow-up at the Rheumatology Service of the Vall d'Hebron Hospital. The sample will be all the patients who attend the clinic routinely for 1 year (July 2022- 23) and who have not been previously diagnosed with ILD. To assess the presence of pulmonary involvement, a detailed history and pulmonary auscultation will be performed. Subsequently, the screening tool will be applied to decide the use of complementary exams if necessary (image 1). The orders of High Resolution Chest CT (HRCTs), lung function tests (LFTs) and chest X-rays are carried out routinely in the regular circuit with the radiology and pulmonology services. <h3>Results</h3> In 6 months (July-December 2023), 349 patients have been screened. Of these, in 22.3% (78) a HRCT request has been recommended, in 31.2% (109) chest X-rays and LFT and in 46.5% (162) regular follow-up. Of the 78 patients to whom HRCT has been recommended, this has already been carried out in 19 patients (the characteristics of these patients are described in Table 1), in two of whom the diagnosis of ILD has been made (1 Non-Specific Interstitial Pneumonia -NSIP- and 1 Usual Interstitial Pneumonia -UIP-), both in mild degree. Neither of the two patients had symptoms or auscultation abnormalities, and both are awaiting LFTs. <h3>Conclusion</h3> The application of this screening algorithm is allowing to diagnose patients with asymptomatic ILD. However, the costs of performing the various complementary exams and its effect in treatment decisions in this profile of patients must continue to be studied. <h3>Reference</h3> [1] Javier Narváez, et al. Criterios de cribado de la enfermedad pulmonar intersticial difusa asociada a la artritis reumatoide: propuesta de expertos basada en metodología Delphi. Reumatología Clínica, 2022. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.
POS1132 HYDROXYCHLOROQUINE, WHICH REGULATES IFN, IS HIGHLY EFFECTIVE IN SLE WITH ELEVATED SERUM S100 PROTEIN
Authors: R. Wakiya, H. Shimada, K. Ueeda, S. Nakashima, T. Miyagi, Y. Ushio, K. Sugihara, R. Mino, M. Mizusaki, K. Chujo, T. Kameda, H. Dobashi
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Background: Serum S100 protein, a damage-associated molecular pattern factor, has been associated with interferon (IFN) [1]. In a previous study, we found that hydroxychloroquine (HCQ), which regulates IFN activity, modulates serum S100 protein in SLE patients [2]. <h3>Objectives</h3> This study determined if the therapeutic effect of HCQ is altered in SLE patients with different serum S100 protein levels. <h3>Methods</h3> This single-center, prospective, exploratory study evaluated patients before HCQ treatment and every 3 months for a year. SELENA-SLEDAI, SLE-DAS, and the Lupus Low Disease Activity State (LLDAS) scale measured disease activity. Serum cytokines (TNF-α, IL-6, IL-8, MCP-1, IL-1ra, VEGF-A, and MIP-1a) were measured by the multiplex Luminex assay, and serum S100A8 and S100A9 protein and adipokines (adiponectin, leptin, and resistin) by ELISA. <h3>Results</h3> Sixty-seven patients (63 females, mean age 41.8 years) were divided into three groups, namely, high, intermediate, and low, according to baseline S100A8 and S100A9 protein levels. The high and low groups were analyzed (Table 1). The S100A8 high group was more likely to have a history of lupus nephritis and active skin lesions. The S100A8 low group had a low serum complement titer (C3) and white blood cell count. The S100A8 high group was more likely to achieve LLDAS after HCQ treatment (high group, 76.9 % vs. low group, 56.3%). SLEDAI and SLE-DAS improved in the S100A8 high group after 3 months of HCQ treatment. There was no significant improvement in SLEDAI and SLE-DAS in the S100A8 low group (Figure 1). The C3 increase was greater in the S100A8 high group after 3 months of HCQ treatment. Regarding serum cytokines and adipokines, serum IL-1ra and resistin levels were significantly higher in the S100A8 high group. Cytokine changes after HCQ treatment were also greater than S100A8 low group. A trend similar to S100A8 was observed in S100A9. <h3>Conclusion</h3> SLE patients with high serum S100 protein levels respond better to HCQ therapy. This finding suggests that serum S100 protein levels may predict the response to IFN inhibitor therapy. <h3>References</h3> [1]Kim JW et al. S100A8 in Serum, Urine, and Saliva as a Potential Biomarker for Systemic Lupus Erythematosus. Front Immunol. 2022;13:886209. [2]Wakiya R et al. Hydroxychloroquine Modulates Elevated Expression of S100 Proteins in Systemic Lupus Erythematosus. Lupus. 2019;28:826–33. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.
AB1448 BISPHOSPHONATES IN (MONA) SPECTRUM DISORDER: TWO CASE REPORTS
Authors: I. Bencharif, D. Bendjenna, Y. Kitouni
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Multicentric osteolysis, nodulosis and arthropathy (MONA) spectrum disorder is a rare inherited progressive skeletal disorder caused by mutations in the matrix metalloproteinase 2 (MMP2) gene. It characterized by multiple peripheral osteolysis, wide metacarpals, osteoporosis, progressive joint contractures, short stature, subcutaneous nodules as well as a coarse face, skin lesions/hirsutism and ocular and cardiac manifestations. The diagnosis is based on the typical clinical features together with normal laboratory findings, which allow this disease to be distinguished from other syndromes, including fibrotic, rheumatic and lysosomal diseases. Treatment options are limited. <h3>Objectives</h3> Bisphosphonates in (MONA) spectrum disorder may be an alternative therapeutic approach. <h3>Methods</h3> We present successful bisphosphonate therapy in two affected sisters, A.N, ten-year-old and A.I, seven-year-old, presented with history of fractures of their long bones after trivial traumas with progressive painful deformities in hands and feet with limitation motion. They were treated with bisphosphonates (intravenous zoledronate every 6 months). The following outcome variables were assessed: skeletal pain, range of motion, internal medical problems as well as neurocognitive function. <h3>Results</h3> Skeletal pain was reduced soon after initiation of therapy. Range of motion did not significantly improve. Neurocognitive development was normal. No further low-impact fractures occurred after starting bisphosphonate therapy. Although the nature of the disease is progressive, they are still able to walk and use their hands for writing. <h3>Conclusion</h3> Bisphosphonate therapy was effective especially in controlling skeletal pain and reducing fractures in MONA spectrum disorder. Early initiation of treatment seems to be particularly important in order to achieve the best possible outcome. <h3>Reference</h3> [1] Pichler, K. et al. Bisphosphonates in multicentric osteolysis, nodulosis and arthropathy (MONA) spectrum disorder – an alternative therapeutic approach. Sci. Rep. 6, 34017; doi: 10.1038/srep34017 (2016) <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.
AB0048 IL-37 ALLEVIATES TNF-Α-INDUCED PYROPTOSIS OF RHEUMATOID ARTHRITIS FIBROBLAST-LIKE SYNOVIOCYTES BY INHIBITING THE NF-ΚB/GSDMD SIGNALING PATHWAY
Authors: C. Ren, Q. Shu, J. Chen, Q. Che, L. Shujie
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Pyroptosis is crucial to rheumatoid arthritis (RA) by inducing and aggravating inflammation. TNF-α is abundant in fibroblast-like synoviocytes of RA (RA-FLSs) and plays a key role in pyroptosis by inducing nuclear factor (NF)-κB activation. Additionally, interleukin (IL)-37 is involved in autoimmune diseases as an anti-inflammatory cytokine and innate and acquired immune response inhibitor. However, the effect of IL-37 on pyroptosis in RA-FLSs remains unclear. <h3>Objectives</h3> To investigate the effects and mechanism of IL-37 on RA-FLS pyroptosis induced by TNF-α and the effect in collagen-induced arthritis (CIA)+TNF model. <h3>Methods</h3> In this study, the serum cytokines in patients with RA and healthy controls were detected using ELISA. The RA-FLSs were then cultured with TNF-α, with or without various IL-37 concentrations, to test the cytokine levels in the cell supernatant. 5-Ethynyl-2′-Deoxyuridine (EdU) assay assessed the effects of IL-37 on RA FLS proliferation. RA-FLS apoptosis was assessed using flow cytometry and mitochondrial membrane potential (MMP) measurement. In addition, transmission electron microscopy (TEM) was used to examine cell pyroptosis. We selected the optimal concentration for the following experiments and detected the signal pathway of IL-37 on pyroptosis of RA FLSs by quantitative reverse-transcription polymerase chain reaction (qRT-PCR) and Western blotting. Finally, we validated the therapeutic effects of IL-37 on CIA rat model in vivo. <h3>Results</h3> IL-37 inhibited inflammation in vitro and in vivo and reduced pyroptosis-related protein expression in RA FLSs. Furthermore, we determined that nuclear factor κB (NF-κB) signaling is required for GSDMD-mediated pyroptosis in RA FLSs. <h3>Conclusion</h3> IL-37 alleviates TNF-α-induced pyroptosis of RA FLSs by inhibiting NF-κB/GSDMD signaling. Additionally, our data revealed a novel mechanism for IL-37 in RA FLSs, suggesting a new potential therapy for IL-37 to treat RA. <h3>REFERENCES:</h3> NIL. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.
AB1186 BODY MASS INDEX EFFECTS QUADRICEPS MUSCLE ARCHITECTURAL CHARACTERISTICS IN PATIENTS WITH KNEE OSTEOARTHRITIS
Authors: E. Ünüvar, N. Cigercioglu, H. Apaydin, H. Guney Deniz
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Sufficient lower extremity muscle mass is necessary for performing functional tasks. It is well known that body mass index (BMI) adversely effect knee cartilage health but in knees with osteoarthritis (OA) it is still unclear how BMI level effect knee muscle architectural characteristics. <h3>Objectives</h3> The aim of this study was to investigate effect of body mass index (BMI) level on quadriceps muscle architecture in patients with knee OA and healthy controls. <h3>Methods</h3> Nineteen patients with OA (mean age= 50.58±4.56 years, mean BMI=28.29±2.51 kg/m2,) and twenty healthy controls (mean age= 48.35±2.83 years, mean BMI=26.76±4.88 kg/m2) were included in the study. Rectus Femoris (RF), Vastus Medialis (VM) and Vastus Lateralis (VL) muscles thickness and pennation angle were evaluated using B-Mode ultrasonography (Esaote MyLab X8 eXP Ultrasound System, Florence, Italy). Ultrasound images were analyzed offline using an image analysis software (Image J, National Institutes of Health, Bethesda, MD, USA) program. When investigating the changes in muscle architecture by group, the effect of BMI was adjusted using covariance analysis (ANCOVA) tests. <h3>Results</h3> There were no differences between age (p=0.073) and BMI (p=0.10) in both groups. Interaction between groupXBMI represented in Table 1. Control group had higher RF muscle thickness, RF pennation angle, VM muscle thickness, VM pennation angle and VL muscle thickness than OA group. There was significant effect of BMI on VM and VL muscle thickness. <h3>Conclusion</h3> Patients with knee osteoarthritis has lower quadriceps muscle thickness and pennation angle than matched healthy controls and BMI was an important factor effecting muscle characteristics. The decrease in VM and VL muscle thickness were associated with increased BMI in knee OA group. These findings highlight the need of body weight control in order to restore muscle thickness in knee OA patients. <h3>References</h3> [1]Aily JB, de Noronha M, de Almeida AC, et al. Evaluation of vastus lateralis architecture and strength of knee extensors in middle-aged and older individuals with knee osteoarthritis. Clin Rheumatol. 2019;38(9):2603-2611. [2]Keng A, Sayre EC, Guermazi A, Nicolaou S, Esdaile JM, Thorne A, Singer J, Kopec JA, Cibere J. Association of body mass index with knee cartilage damage in an asymptomatic population-based study. BMC Musculoskelet Disord. 2017 Dec 8;18(1):517. doi: 10.1186/s12891-017-1884-7. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.
AB0381 TREATMENT EXPECTATIONS OF PATIENTS WITH RHEUMATOID ARTHRITIS BEFORE STARTING METHOTREXATE
Authors: U. Kiltz, A. Petridou, N. Timmesfeld, W. Rief, K. Schmidt, D. Krause, U. Bingel, J. Braun
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Although the long-term efficacy, tolerability, and safety of methotrexate (MTX) in patients with rheumatoid arthritis (RA) are well documented, the clinical response and incidence of adverse events (AE) vary. Data on adherence to MTX in RA patients vary between retention rate of 35% to 80% in 5 years. In addition to aspects of safety, important determinants for continuing MTX therapy are patient-associated factors including individual treatment preferences and expectations. <h3>Objectives</h3> This study investigated treatment expectations in RA patients who were about to start therapy with MTX, and aim to identify variables associated with discontinuation of MTX. <h3>Methods</h3> Consecutive patients with RA who were about to start MTX were prospectively included after informed consent. Treatment expectations, medication-related beliefs and AE, treatment satisfaction and health–related quality of life were assessed using the generic rating scale for previous experiences, expectations and effects of treatment (G-EEE), the general assessment of side effects (GASE), the beliefs about medicines questionnaire (BMQ), the treatment satisfaction questionnaire for medication (TSQM) and the EuroQol-5 dimensions (EQ-5D) before treatment initiation (T0), and after 3 months (T1). Treatment adherence to MTX was checked at month 6 (T2). Associations treatment expectations and adherence to MTX were explored by regression analyses. <h3>Results</h3> A total of n=100 consecutive RA patients before starting MTX treatment were included (table 1). A history of inadequate response to sulfasalazine was determined in n=6 (6%) and to hydroxychloroquine in n=1 patient, respectively. At T0, patients perceived the need for treatment with MTX as high, and they were only moderately concerned regarding potential adverse events of this therapy. Treatment expectations regarding the decrease of pain levels and development of adverse events did not differ much between T0 and T1, when disease activity had significantly decreased and physical function increased. MTX was withdrawn in 3 out of 59 patients (5.0%) and in 13 out of 64 patients (20.3%) assessed at T1 and T2, respectively, with a mean of 16.0 (5.9) weeks after treatment initiation. The most frequent reason for discontinuation of MTX was the occurrence of adverse events (n=12) followed by infections or malignancies (both n=2). There was no significant relationship between patients' treatment expectations reported at T0 and their adherence to MTX at T2. <h3>Conclusion</h3> In this study, we found no evidence for the assumption that patients' expectations before the start of MTX did substantially influence adherence to treatment. <h3>REFERENCES:</h3> NIL. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.
POS1552 NO BURN-OUT IN PEDIATRIC-ONSET BEHÇET’S DISEASE PATIENTS DURING ADULTHOOD FOLLOW-UP
Authors: T. Bozkurt, M. Karabacak, H. Karataş, S. Kutluğ Ağaçkiran, T. Ergun, H. Direskeneli, F. Alibaz-Oner
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Although onset of Behçet's Disease (BD) is common in the second or third decade, initial symptoms occur under the age of 16 years in 4–26% of the patients [1]. <h3>Objectives</h3> In this study we aimed to assess the clinical course of pediatric onset BD in adulthood period. <h3>Methods</h3> The files of 1114 BD patients were reviewed retrospectively. 51 (4.6%) (F/M:21/30) pediatric-onset BD patients were included in the analysis. Demographic and clinical characteristics, follow-up and treatment data of the patients were recorded from files. <h3>Results</h3> The median age at diagnosis was 16 (14-17) years, and the median follow-up duration was 51 (26-96) months. Of all patients, 33.3% (n=17) had ocular involvement, 27.5% (n=14) had vascular involvement, 17.6% (n=9) had central nervous system involvement, and 3.9% (n=2) had gastrointestinal system involvement. Erythema nodosum was more common in females (p=0.008) and vascular involvement was more common in males (p=0.025). At the end of follow-up, 32 (62.8%) patients had major organ involvement, half of them had at the time of diagnosis. Forty-seven (M:27/F:20) patients had a follow-up with median of 50 (20-82) months in adulthood period. Thirty-one (65.6%) patients had major organ involvement. While 20 (64.5%) patients had major organ involvement in the pediatric period, 11 (35.5%) patients developed major organ involvement in adulthood. Overall, 19 (40.4%) patients had active disease manifestations (relapse and/or new major organ involvement) in adulthood follow-up. Of these patients, 11 (57.9%) had new major organ involvement, 7 (36.8%) had a relapse of the same organ, and one (5.3%) had both new major organ involvement and a relapse. The disease course of patients is seen in Table 1. <h3>Conclusion</h3> Our results show that, about half of the pediatric-onset BD patients still have active disease manifestations (mainly new major organ involvement) in adulthood period. <h3>Reference</h3> [1]Yildiz, M., et al., Pediatric Behçet's disease-clinical aspects and current concepts. European journal of rheumatology, 2020. <b>7</b>(Suppl 1): p. S38. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.
AB1495 THE RELATIONSHIP BETWEEN AMYLOIDOSIS AND ATHEROSCLEROSIS IN FAMILIAL MEDITERRANEAN FEVER
Authors: S. Gür, V. Yazisiz, M. E. Terzioğlu, F. Erbasan
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Familial Mediterranean Fever (FMF) is an autosomal recessive autoinflammatory disease characterized by recurrent fever and serosal inflammation signs [1]. The most severe complication of the disease is AA amyloidosis, with increased mortality and morbidity [2]. <h3>Objectives</h3> To determine the relationship between amyloidosis and atherosclerosis in FMF patients. <h3>Methods</h3> 730 FMF patients were analyzed retrospectively for the development of amyloidosis and coroner artery disease (CAD). Cut-off values of serum amyloid A (SAA), fibrinogen, c-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) variables that could predict amyloidosis and CAD were evaluated by receiver operating characteristic (ROC) analysis. <h3>Results</h3> The prevalences of amyloidosis and CAD were 6% and 3.7%, respectively. The incidence of CAD was higher in patients with amyloidosis (13.6%) than in patients without amyloidosis (3.1%) (p=0.004). Cut-off values that could predict amyloidosis were 44 ng/ml for SAA, 384 mg/dl for fibrinogen, 20 mm/hour for ESR, and 1.25 mg/dl for CRP (Table 1). The adjusted odds ratio for amyloidosis was 5.5-fold in patients with SSA &gt;44 ng/ml and 3.4 in patients with ESR &gt;20 mm/hour (Table 1). Serum AA level was significantly higher in patients with CAD (85,4±118,9) than in the group without CAD (32,4±49,5) (p=0.008). The area under the curve (AUC) values of SAA, CRP, ESR, and fibrinogen were not statistically significant for predicting CAD (Fig 1). <h3>Conclusion</h3> The risk of CAD is increased in amyloidosis secondary to FMF disease. <h3>References</h3> [1]Shohat, M., Halpern, G. Familial Mediterranean fever—A review. Genet Med 13, 487–498 (2011). https://doi.org/10.1097/GIM.0b013e3182060456. 2[] van der Hilst JC, Simon A, Drenth JP. Hereditary periodic fever and reactive amyloidosis. Clin Exp Med. 2005 Oct;5(3):87-98. doi: 10.1007/s10238-005-0071-6. <h3>Disclosure of Interests</h3> None Declared. <h3>Disclosure of Interests</h3> None Declared.